PRACTICAL NEUROLOGY最新文献

A 39-year-old woman presented with headaches, slurred speech, facial weakness and brief altered sensations, together with non-specific visual disturbances, unsteadiness and falls. Despite initial treatment with intravenous methylprednisolone, her condition worsened with cognitive decline, confusion, ataxia and incontinence. We diagnosed Susac's syndrome having excluded other causes. Her management was difficult, taking 4 months to achieve remission with corticosteroids, rituximab, cyclophosphamide and intravenous immunoglobulin (Ig). She continues to be on aspirin, intravenous Ig and mycophenolate mofetil to maintain remission, with cyclophosphamide planned for possible relapses.

The most common ictal psychiatric symptom is fear, followed by ictal depression, and least commonly ictal psychosis. A 44-year-old man with poststroke focal epilepsy developed new-onset behavioural change, disorientation, hallucinations and delusions, persisting for several months before medical evaluation. He was agitated, disorientated, with impaired memory and disorganised thinking, but had no minor epileptic motor signs. His EEG showed electroclinical status epilepticus. His psychotic symptoms persisted despite seizure control and required antipsychotic treatment. Ictal psychosis is rare but clinicians should suspect this in people with epilepsy with new-onset behavioural change. Rarely, it can persist for months, sometimes needing antipsychotic treatment.

Status epilepticus is a serious neurological emergency requiring swift recognition and treatment. Presentations with prominent motor features are easily recognised but it can be challenging to diagnose those with subtle or no motor features. Electroencephalogram (EEG) remains indispensable in diagnosing, classifying, monitoring and prognosticating of status epilepticus. There are several separate classification systems for seizures, epilepsy and status epilepticus, incorporating clinical features, causes and EEG correlates. This review focuses on using EEG in status epilepticus and provides a practical approach to diagnosis and classification aligning with the current International League Against Epilepsy and American Clinical Neurophysiology Society definitions.

Inflammation of the pituitary gland can be primary (without another underlying cause) or secondary (associated with a systemic inflammatory condition). Primary hypophysitis is very rare, among which xanthomatous hypophysitis as a histological type is extremely unusual. A woman in her late 50s presented with recurrent pituitary lesions over 20 years. Her general practitioner had diagnosed panhypopituitarism in her 30s; a decade later, she had presented to ophthalmology with visual loss and restricted visual fields, and a pituitary lesion was found. This recurred several times requiring multiple resections. Histopathology showed atypical inflammation in keeping with xanthomatous hypophysitis; this responded well to corticosteroid therapy. Xanthomatous hypophysitis is a rare form of steroid-responsive primary pituitary inflammation, to consider in the differential diagnosis of recurring pituitary lesions.

Most minor head injuries have no immediate neurological sequelae. We present a case where acute neurological symptoms followed a very minor head injury, and an underlying genetic cause was identified. We highlight the role that head injuries, even when innocuous, may have in precipitating and worsening a neurogenetic disorder.